SYSTEMIC AND PULMONARY VENOUS CONNECTIONS IN VISCERAL HETEROTAXY WITHASPLENIA - DIAGNOSTIC AND SURGICAL CONSIDERATIONS BASED ON 72 AUTOPSIED CASES

To facilitate the preoperative diagnosis and surgical management of vi sceral heterotaxy and asplenia, 72 postmortem cases were reviewed with particular attention focused on the systemic and pulmonary venous con nections, The superior vena cava was bilateral in 51 cases (71%), but in 9 cases one of the superior venae cavae was partly or totally atret ic, Patent bilateral superior venae cavae were found in 42 cases (58%) and the superior vena cava was unilateral in 21 (29%), Although the i nferior vena cava was never interrupted, a prominent azygos vein was f ound in 6 cases (8%), Some hepatic veins drained separately from the i nferior vena cava in 20 cases (28%), An intact coronary sinus was rare (2 cases, 3%), Anomalous pulmonary venous connection to a systemic ve in was total in 42 (58%) of 72 and partial in 2 (3%) of 72, with obstr uction in 24 (55%) of 44, Abnormal pulmonary artery branches (severe h ypoplasia, localized stenosis, or discontinuity) were present in 21 (2 9%), and these obstructive arterial anomalies were associated with a s ignificantly higher prevalence of anomalous pulmonary venous connectio n (p < 0.01) and of pulmonary venous obstruction (p < 0.01), Cardiac p ulmonary venous connections were found in 28 (39%), with the pulmonary veins and the inferior vena cava entering the same atrium in 10 (36%) of 28.